Diseases Diseases

How To Treat a Patient With Lissencephaly

Introduction

Lissencephaly, which literally means smooth brain, is a rare brain disorder. It is caused by defective neuronal migration during the 12th and 14th week of gestation by the mother. The normal structure of the brain is supposed to have folds and grooves on the grey matter’s surface or on the cerebrum. However, during pregnancy the structure of the brain did not develop well thus the brain of the fetus was poorly developed resulting to smooth surfaced brain structure. Lissencephaly likely developed during pregnancy due to injury during pregnancy, mother’s intake of teratogenic or harmful for pregnancy drugs, any form of viral infection by the mother or insufficient blood supply provided by the mother to the baby through the umbilical cord. Unfortunately, lissencephaly is untreatable. However, we can lead the treatment to symptomatic relief which depends on the severity and locations of the brain malformations. Supportive care is needed for the patient to assist them in comfort and nursing needs. Prognosis of lissencephaly depends on the severity of the brain malfunction. Many patients who suffer from this disease show no significant change of development during 3-5 months of life of the infant. Because of modern medications and therapy, some children can live through their teens as long as symptoms have been relieved well. So how can we support patients with lissencephaly? Here’s a 5-step guide on how to take care of these patients.

Step 1

Know what Lissencephaly looks like:

You have to recognize what the signs and symptoms of lissencephaly are. If your child manifests any form of seizure disorder, difficulty swallowing or eating, ataxic gait or spastic legs, slowed growth and developmental delay, and/or mental retardation, it’s time to consult your doctor about these manifestations. These are some of the common manifestations of lissencephaly which need immediate attention by physician. In addition to symptoms, some children might also have deformed hands, fingers and toes.

Step 2

Lissencephaly must be properly diagnosed:

Lissencephaly can be detected through proper diagnosis. Contact a pedia-neurologist and have you child scheduled for a check-up. Lissencephaly can be diagnosed through magnetic resonance imaging (MRI) or computed tomography scan (CT scan).

Step 3

Assist lissencephaly patients with their daily activities of living:

Help with their daily activities of living such as nutrition needs, toileting needs and hygienic needs. Patients with lissencephaly grow to be dependent throughout their lifetime, depending on the severity of the brain malfunction. Be patient with these individuals. This requires huge amount of patience and understanding on the part of the caregiver.

Step 4

Control the symptoms of patients with lissencephaly


Now that you know the classic signs and symptoms of lissencephaly, you can help your patient by minimizing the symptoms. Ask your doctor on how to properly manage these symptoms. If your patient suffers from a seizure, do not control your patient’s seizure movement. Have your patient lie down on a flat surface, turn the head to side and put a padded tongue depressor as soon as an aura of a seizure has been spotted. If your patient is having difficulty swallowing or eating, liquefy foods to allow easier swallowing. If swallowing difficulty is severe, gastrostomy tube may be required to facilitate nutritional needs.

Step 5

Provide generous amounts of emotional care in lissencephaly patients:

Basic treatment of lissencephaly shouldn’t be focused on the physical aspect but as well as emotional aspect. These patients need to know that despite their condition, they should feel that they are loved and appreciated.

By Seth Evan, published at 02/22/2012
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